Primary IVC Leiomyosarcoma: A Case Series and Review of the Literature

Abstract

Leiomyosarcomas of the inferior vena cava (IVC) are the most common primary malignancy of the IVC; however, these lesions are extremely rare. These lesions are slow-growing, often asymptomatic and, present late in the disease course portending a poor prognosis. Surgical resection is considered the standard of care and only potential curative treatment. Progression free survival rates after surgical resection have been estimated to be 31.4% after five years and 7.4% after 10 years. To date, there have been a total of 418 cases documented within small and large single center institutional case series. Here, we present a case series from a single institution and a comprehensive literature review to investigate the presentation, treatment, and outcomes of this rare lesion. A surgically relevant designation of tumor location to include involvement of the renal veins is proposed.

Keywords: clinical presentation; inferior vena cava; leiomyosarcoma; primary venous malignancy; resection; surgery.